Pediatric Cardiology

, Volume 29, Issue 2, pp 309–312

Pulmonary Hypertension in Children and Adolescents with Sickle Cell Disease

Authors

    • Center for Sickle Cell DiseaseHoward University
  • A. Campbell
    • Center for Sickle Cell DiseaseUniversity of Michigan
  • M. Teshome
    • Center for Sickle Cell DiseaseUniversity of Michigan
  • S. Onyeagoro
    • Center for Sickle Cell DiseaseHoward University
  • C. Sylvan
    • Center for Sickle Cell DiseaseHoward University
  • A. Akintilo
    • Center for Sickle Cell DiseaseHoward University
  • S. Hutchinson
    • Center for Sickle Cell DiseaseUniversity of Michigan
  • G. Ensing
    • Center for Sickle Cell DiseaseUniversity of Michigan
  • P. Gaskin
    • Department of Pediatrics and Child HealthHoward University
    • Pediatric CardiologyUniversity of Maryland
  • G. Kato
    • Vascular Therapeutics Section, National Heart, Lung and Blood Institute, and the Critical Care Medicine DepartmentClinical Center, National Institutes of Health
  • S. Rana
    • Department of Pediatrics and Child HealthHoward University
  • J. Kwagyan
    • General Clinical Research CenterHoward University
  • V. Gordeuk
    • Center for Sickle Cell DiseaseHoward University
    • General Clinical Research CenterHoward University
  • J. Williams
    • Center for Sickle Cell DiseaseUniversity of Michigan
  • O. Castro
    • Center for Sickle Cell DiseaseHoward University
Article

DOI: 10.1007/s00246-007-9018-x

Cite this article as:
Onyekwere, O.C., Campbell, A., Teshome, M. et al. Pediatr Cardiol (2008) 29: 309. doi:10.1007/s00246-007-9018-x

Abstract

The prevalence of pulmonary hypertension (PHTN) in the pediatric sickle cell disease (SCD) population is not known despite its high prevalence in adult patients. Our hypothesis was that increased pulmonary artery pressures (PAPs) would be found in SCD children and adolescents, especially those with a history of pulmonary complications: acute chest syndrome, obstructive sleep apnea, asthma, and reactive airway disease. Fifty-two SCD children, 23 of whom had underlying pulmonary disease, were screened for PHTN, which was defined as a tricuspid regurgitant jet velocity (TRV) of at least 2.5 m/s. Twenty-four (46.15%) SCD patients had increased PAP (i.e., TRV ≥2.5 m/s), and 6 (11.5%) had significant PHTN (i.e., TRV ≥3.0 m/s). Pulmonary disease was marginally associated with PHTN (odds ratio 2.80 and confidence interval 0.88 to 8.86; p = 0.0795). As in adult SCD patients with PHTN, this complication was correlated with the degree of hemolysis as manifested by significantly higher lactate dehydrogenase and bilirubin, lower hemoglobin and hematocrit levels, and a strong association with Hb-SS phenotype. However, after statistical adjustment for age and sex, increased serum LDH was not associated with the development of PHTN. Further studies are needed to clarify the prevalence and mechanisms of PHTN in pediatric and adolescent patients with SCD.

Keywords

HemolysisPulmonary diseaseSickle cell diseaseTricuspid valve regurgitation

Copyright information

© Springer Science+Business Media, LLC 2007